Prediction of intravenous immunoglobulin resistance in patients with Kawasaki disease according to the duration of illness prior to treatment.

Risk factors predicting intravenous immunoglobulin (IVIG) resistance in patients with Kawasaki disease (KD) were assessed according to the duration of illness prior to treatment. Of 555 KD patients included between 2008 and 2014, 362 were IVIG responders (65.2%) and 193 were IVIG non-responders (34.

Molecular diagnosis of hereditary spherocytosis by multi-gene target sequencing in Korea: matching with osmotic fragility test and presence of spherocyte.

Background: Current diagnostic tests for hereditary spherocytosis (HS) focus on the detection of hemolysis or indirectly assessing defects of membrane protein, whereas direct methods to detect protein defects are complicated and difficult to implement. In the present study, we investigated the patterns of genetic variation associated with HS among patients clinically diagnosed with HS.

Methods: Multi-gene targeted sequencing of 43 genes (17 RBC membrane protein-encoding genes, 20 RBC enzyme-encoding genes, and six additional genes for the differential diagnosis) was performed using the Illumina HiSeq platform.

Clinical and laboratory profiles of hospitalized children with acute respiratory virus infection.

Purpose: Despite the availability of molecular methods, identification of the causative virus in children with acute respiratory infections (ARIs) has proven difficult as the same viruses are often detected in asymptomatic children.

Methods: Multiplex reverse transcription polymerase chain reaction assays were performed to detect 15 common respiratory viruses in children under 15 years of age who were hospitalized with ARI between January 2013 and December 2015. Viral epidemiology and clinical profiles of single virus infections were evaluated.

B7‑H1‑mediated immunosuppressive properties in human mesenchymal stem cells are mediated by STAT‑1 and not PI3K/Akt signaling.

Mesenchymal stem cells (MSCs), derived from either bone marrow (BM) or Wharton's jelly (WJ), inhibit the proliferation of activated T cells, and interferon (IFN)‑γ serves an important role in this process. This process is B7‑homolog (H)1‑dependent during cell contact inhibition. However, the signaling pathway involved in B7‑H1 expression in MSCs remains largely undefined.

Laboratory Markers in Incomplete Kawasaki Disease according to Coronary Artery Outcome.

Background And Objectives: We defined laboratory marker profiles typical of incomplete Kawasaki disease (iKD) during illness, especially with respect to the presence of a coronary artery abnormality such as coronary artery dilation or aneurysm.

Methods: This retrospective study examined the clinical and laboratory markers of patients with iKD over time, along with those of patients with complete KD (cKD) and febrile controls.

Results: Of 795 patients, 178 had iKD, 504 had cKD and 113 were febrile controls.

Systemic Juvenile Xanthogranuloma Involving the Bone Marrow, Multiple Bones, and the Skin That Developed During Treatment of Acute Lymphoblastic Leukemia in Remission State.

Juvenile xanthogranuloma (JXG) is a rare benign disorder classified as non-Langerhans cell histiocytosis, with unclear etiology and pathogenesis. JXG is generally characterized by solitary or multiple cutaneous nodules that resolve spontaneously over a few years. JXG rarely presents as extracutaneous lesions that progress to a symptomatic systemic disorder through multiple organ involvement.

Familial Mediterranean fever presenting as fever of unknown origin in Korea.

Familial Mediterranean fever (FMF) is the most common Mendelian autoinflammatory disease, characterized by uncontrolled activation of the innate immune system that manifests as recurrent brief fever and polyserositis (e.g., peritonitis, pleuritic, and arthritis).

Characteristics and Outcomes of Second Malignant Neoplasms after Childhood Cancer Treatment: Multi-Center Retrospective Survey.

This retrospective study investigated the clinical characteristics and outcomes of second malignant neoplasms (SMNs) in survivors of childhood cancer from multiple institutions in Korea. A total of 102 patients from 11 institutions who developed SMN after childhood cancer treatment between 1998 and 2011 were retrospectively enrolled. The most common primary malignant neoplasms (PMNs) were central nervous system (CNS) tumors (n = 17), followed by acute lymphoblastic leukemia (n = 16), non-Hodgkin lymphoma (n = 13), and osteosarcoma (n = 12).

Value of neutrophil-lymphocyte ratio in predicting outcomes in Kawasaki disease.

Total and differential leukocyte counts are useful inflammatory biomarkers. The ability of the neutrophil-to-lymphocyte ratio (NLR) to predict outcomes in patients with Kawasaki disease (KD) was assessed in this study. All patients with KD who underwent consecutive complete blood count analyses during the acute febrile phase before intravenous immunoglobulin (IVIG), 2 days after IVIG regardless of defervescence, and 3 to 4 weeks after defervescence were enrolled.

The association between influenza treatment and hospitalization-associated outcomes among Korean children with laboratory-confirmed influenza.

There are limited data evaluating the relationship between influenza treatment and hospitalization duration. Our purpose assessed the association between different treatments and hospital stay among Korean pediatric influenza patients. Total 770 children ≤ 15 yr-of-age hospitalized with community-acquired laboratory-confirmed influenza at three large urban tertiary care hospitals were identified through a retrospective medical chart review.

Clinical features and treatment outcomes of Langerhans cell histiocytosis: a nationwide survey from Korea histiocytosis working party.

A nationwide survey was conducted to clarify the clinical features and outcomes of Korean children with Langerhans cell histiocytosis (LCH). Korea Histiocytosis Working Party analyzed the data of 603 patients who were diagnosed with LCH between 1986 and 2010 from 28 institutions in Korea. Median age at diagnosis was 65 months (range, 0 to 276 mo).

Rapid growing superficial cutaneous leiomyosarcoma of the face.

Leiomyosarcomas are uncommon malignant smooth muscle tumors, mainly derived from vessels or viscera. Superficial leiomyosarcomas are a rare soft tissue sarcoma arising from the dermis or subcutaneous tissue in the skin. According to tumor origin and location, they are divided into cutaneous and subcutaneous leiomyosarcoma.

Quantitative comparison of CDKN2B methylation in pediatric and adult myelodysplastic syndromes.

Background/aims: Transcriptional repression of tumor suppressor genes is determined by the quantity of promoter hypermethylation. We analyzed the methylation quantity of CDKN2B in pediatric myelodysplastic syndromes (MDS).

Methods: Quantitative measurement of CDKN2B methylation was performed in 25 pediatric MDS patients and 12 controls using pyrosequencing, and the result was compared with those from 74 adult MDS cases and 31 adult controls.

Idiopathic acute eosinophilic pneumonia in a 14-month-old girl.

Idiopathic acute eosinophilic pneumonia (IAEP), characterized by acute febrile respiratory failure associated with diffuse radiographic infiltrates and pulmonary eosinophilia, is rarely reported in children. Diagnosis is based on an association of characteristic features including acute respiratory failure with fever, bilateral infiltrates on the chest X-ray, severe hypoxemia and bronchoalveolar lavage fluid >25% eosinophils or a predominant eosinophilic infiltrate in lung biopsies in the absence of any identifiable etiology. We present a 14-month-old girl who was admitted to our pediatric intensive care unit because of acute respiratory distress.

Incomplete clinical manifestation as a risk factor for coronary artery abnormalities in Kawasaki disease: a meta-analysis.

Incomplete Kawasaki disease (KD) comprises a large proportion of the total number of cases. Although it has the potential of delaying diagnosis, it is not conclusive whether an incomplete presentation is a risk factor for coronary artery abnormalities (CAAs). We performed a meta-analysis to establish the risk of CAA in 20 studies including 4,504 cases and 32,519 controls, and the risk of giant aneurysm in two studies including 5,390 cases and 37,648 controls.

Alteration of CD4CD25Foxp3 T cell level in Kawasaki disease.

Purpose: Exaggerated pro-inflammatory reactions during the acute phase of Kawasaki disease (KD) suggest the role of immune dysregulation in the pathogenesis of KD. We investigated the profiles of T regulatory cells and their correlation with the clinical course of KD.

Methods: Peripheral blood mononuclear cells were collected from 17 KD patients during acute febrile and subacute afebrile phases.

Epidemiology and clinical long-term outcome of childhood aplastic anemia in Korea for 15 years: retrospective study of the Korean Society of Pediatric Hematology Oncology (KSPHO).

Purpose: Aplastic anemia (AA) is a rare hematologic disease characterized by pancytopenia and hypocellular marrow. The Korean Society of Pediatric Hematology Oncology investigated retrospectively the incidence, survival, and transfusion independency according to treatment strategies in AA.

Methods: All the questionnaires were sent to members for medical records.

Altered cytokine profiles of mononuclear cells after stimulation in a patient with Blau syndrome.

Autoinflammatory Blau syndrome (BS) is associated with NOD2 gene mutations that lead to constitutive NFkappaB activation. NOD2 functions as an intracellular receptor for the muramyl dipeptide (MDP) component of peptidoglycan (PGN). The objectives of this study are to analyse whether NFkappaB activation in BS affects immune cell functions, and whether NOD2 and toll-like receptor (TLR) pathways interact.

Increased serum interleukin-5 and vascular endothelial growth factor in children with acute mycoplasma pneumonia and wheeze.

Acute mycoplasma pneumonia may be accompanied by wheeze in some children considered not to have asthma. The aim of the present study was to evaluate cytokine secretion in children with acute mycoplasma pneumonia and wheeze. We studied 58 patients with mycoplasma pneumonia (12 with wheeze, Group 1; 46 without wheeze, Group 2) and 36 patients of non-mycoplasma pneumonia (Group 3).

Risk factors for Kawasaki disease-associated coronary abnormalities differ depending on age.

Introduction: The clinical manifestations and risk factors for developing coronary artery abnormalities (CAA) in Kawasaki disease (KD) might differ depending on age.

Materials And Methods: From January 2001 to July 2007, 161 patients with an age younger than 1 year (younger group) and 60 patients with an age older than 5 years (older group) were diagnosed with KD at the Korea University Medical Center. Their medical records were reviewed retrospectively and the two groups were compared in terms of a number of variables commonly associated with the development of CAA, including clinical manifestations and laboratory findings.

Incomplete Kawasaki disease in patients younger than 1 year of age: a possible inherent risk factor.

Kawasaki disease (KD) patients younger than 1 year of age are at especially high risk of developing coronary artery abnormalities (CAA). To define the clinical characteristics of this group, as well as the risk factors predisposing them to CAA, we reviewed the medical records of 136 KD patients younger than 1 year of age who were treated at the Korea University Medical Center from January 2001 to July 2006. Of these patients, 16 developed CAA (11.

Immunogenicity and safety of Vi capsular polysaccharide typhoid vaccine in healthy persons in Korea.

The purpose of this study was to evaluate the immunogenicity and safety of Salmonella Typhi Vi capsular polysaccharide vaccine (Vi vaccine) in Korea. The immunogenicity of a single dose of Vi vaccine was evaluated in 157 subjects (75 children and 82 adults) before and at 1, 6, and 12 months after vaccination. Immunogenicity was measured with a passive hemagglutination assay (PHA), quantified as geometric mean titers (GMTs) and seroconversion rates.

Use of RNA interference to elucidate the effect of MYCN on cell cycle in neuroblastoma.

Background: MYCN amplification marks poor prognosis in neuroblastoma (NB) tumors. In evaluating the mechanisms by which retinoic acid (RA) or nerve growth factor (NGF) decrease cell number in MYCN amplified NB cells, we have identified a number of proteins whose expression either decreases (E2F, CDC2, CDK6, cyclin dependent kinase activity) or increases (p27) in association with a decrease in MYCN expression. However, it was still unclear which were MYCN dependent effects or not.